کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4171431 1275657 2011 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Respiratory Manifestations in Mucopolysaccharidoses
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی پریناتولوژی (پزشکی مادر و جنین)، طب اطفال و بهداشت کودک
پیش نمایش صفحه اول مقاله
Respiratory Manifestations in Mucopolysaccharidoses
چکیده انگلیسی

SummaryMucopolysaccharidoses (MPS) are a group of inherited, metabolic diseases caused by deficiency of lysosomal enzymes that degrade glycosaminoglycans (GAG). Loss of enzyme activity results in cellular accumulation of GAG fragments leading to the progressive multi-system manifestations. MPS are classified into seven clinical types based on eleven known lysosomal enzyme deficiencies of GAG metabolism. Respiratory involvement is seen in most MPS types with recurrent respiratory infections, upper and lower airway obstruction, tracheomalacia, restrictive lung disease, and sleep disturbances. Patients with airway obstruction are at high risk for anaesthetic complications. In this review, we present the respiratory manifestations in various MPS types and stages, evaluation of respiratory involvement, and treatment options for the progressive respiratory failure that occurs in MPS patients.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Paediatric Respiratory Reviews - Volume 12, Issue 2, June 2011, Pages 133–138
نویسندگان
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